J Neurol (2012) 259:1561–1565 DOI 10.1007/s00415-011-6376-3
ORIGINAL COMMUNICATION
Hemifacial spasm: clinical characteristics of 321 Indian patients Amit Batla • Chanchal Goyal • Garima Shukla • Vinay Goyal • Achal Srivastava • Madhuri Behari
Received: 13 October 2011 / Revised: 6 December 2011 / Accepted: 11 December 2011 / Published online: 6 January 2012 Ó Springer-Verlag 2011
Abstract Hemifacial spasm (HFS) is a common neurological disorder characterized by involuntary tonic and clonic contractions of the muscles innervated by the facial nerve. We aimed to describe clinical features, common antecedents, triggers and relieving factors in patients with hemifacial spasm to study the correlation of hypertension and HFS, and to compare clinical features of primary and secondary cases of HFS. The data for the study were collected prospectively on a predesigned and pre-tested format at the first attendance in all consecutive HFS patients attending the movement disorders clinic of a tertiary teaching hospital in India. The demographic profile, HFS symptoms, antecedent illnesses and neurological examination were recorded and analyzed. Muscle power in individual muscles innervated by the facial nerve was tested carefully before botulinum toxin injection. Hemifacial spasm occurred in 7.14% (n = 582) of 8,151 cases registered at the movement disorders clinic from 1993 to 2010. Data of 321 patients were complete and were included in the study. Females constituted 49.22% (n = 158). The mean age the patients was 46.02 ± 11.82 years; ipsilateral ear clicking was observed in 22.74% cases. The most common aggravating factor was stress (44.86%), while the most common relieving factor was sleep (44.24%). Two hundred fifty-two patients (78.5%) had primary HFS. The severity of spasm correlated significantly with disease duration (p \ 0.001) and weakness of facial muscles (p \ 0.001). We did not
A. Batla C. Goyal G. Shukla V. Goyal A. Srivastava M. Behari (&) Department of Neurology, All India Institute of Medical Sciences, New Delhi 110029, India e-mail:
[email protected]
observe any correlation between HFS on the left side and hypertension, as has been reported earlier. This is one of the largest studies of HFS patients and the only one that prospectively assesses patients with HFS clinically on their first visit. Interesting observations of this study are lack of female preponderance, presence of clicking in the ipsilateral ear and facial weakness even prior to botulinum toxin injection. Keywords Hemifacial spasm Facial nerve Facial weakness Ear clicking
Introduction Hemifacial spasm (HFS) is characterized by involuntary tonic and clonic contractions of the muscles innervated by the facial nerve. This may be secondary to earlier involvement of the VIIth nerve or idiopathic (primary HFS) when no cause is apparent. Primary HFS probably results from hyperactivity of the facial nucleus due to ephaptic transmission resulting from compression by an aberrant vascular loop on the VIIth cranial nerve as it exits the brainstem [1–6]. Hypertension is reported to be associated with left-sided HFS when there is neurovascular compression on the left side of the ventrolateral medulla (VLM) at the root-exit zone of cranial nerves IX and X [7]. The prevalence of HFS was estimated to be 14.5 per 100,000 in women and 7.4 per 100,000 in men in one large community-based study in the US [8]. It has been reported to be more common among Asians living in the US with 3.1 times higher frequency as compared to cranio–cervical dystonia [9]. Hemifacial spasm is more common in females [10] and is associated with mild facial weakness [10, 11]. The weakness may follow botulinum toxin injection too,
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which is a common form of HFS therapy. It is more common in cases following Bell’s palsy because of mild residual weakness. Hearing loss is common in secondary HFS cases [11, 12] presumably due to associated middle ear involvement leading to VIIth nerve palsy. The disorder is chronic, and the clinical features are often modified with treatment. No large studies have analyzed the clinical features prior to treatment initiation. In this study, we assessed clinical features of HFS at the first hospital visit of these patients. Study objectives The goals of the study were to document clinical features, common antecedents, association with hypertension, and aggravating and relieving factors in HFS patients at their first evaluation, and to: 1. 2.
Study the association of hypertension with the side of HFS, Compare the clinical features of primary and secondary cases of HFS
Methods The data for the study were collected prospectively on a predesigned and pre-tested format at the first attendance in all consecutive HFS patients attending the movement disorders clinic of a tertiary teaching hospital in India seen from 1993 to 2010 and transcribed on to an Excel spreadsheet. The following information was collected: demographic profile, details of HFS symptoms, associated symptoms, antecedent illnesses and neurological examination. The power of individual facial muscles was tested before botulinum toxin injection. Hypertension defined according to JNC 7 was used to assess its association with clinical features. MRI brain was done when indicated, and the results were recorded. Severity of the HFS was scored on a 5-point Likert scale from 0 to 4 (0 = not affected; 4 = very severe, prolonged spasm). The study was undertaken after approval of the institutional ethics committee and receiving informed consent from the patients prior to inclusion in the study. Statistical analysis Statistical analysis was carried out using STATA 9.0 (College Station, TX, USA). Chi-square test, t test and Wilcoxon rank sum test were applied to calculate any significant differences between groups. The odds ratio (95% confidence interval) was calculated for the factors
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that showed significant differences. A p value of \0.05 was considered statistically significant.
Results Hemifacial spasm occurred in 7.14% of a total of 8,151 patients registered at the movement disorders clinic during the study period (1993–2010). Of the 582 HFS patients, complete records were available for 321 patients, and these were included for analysis. The mean ± SD age of the patients was 46.02 ± 11.82 years, while the mean age of onset was 42.60 (SD = 11.77) years and the mean duration of HFS was 43.14 months (range 1–300 months). Females constituted 49.22% (n = 158) of all HFS patients (Table 1). The upper face (orbicularis oculi) was affected initially more often (68.85%) compared to the lower face (5.29%), and the mean (SD) interval between involvement of the upper and lower half of the face was 19.28 (22.81) months. One hundred patients (31.15%), however, reported involvement of both the lower and upper half of the face simultaneously. Hearing loss was present in 11.21% of cases and was more often seen in secondary HFS cases (p \ 0.001). Clicking in the ipsilateral ear was observed in 22.74% of all the cases. The most common aggravating factor was mental stress (44.86%) followed by speaking (37.96%), while the most common relieving factor was sleep (44.24%). Two hundred fifty-two patients (78.5%) had primary HFS, and 69 patients (21.5%) had the secondary form. Causes of secondary HFS were Bell’s palsy (n = 27, 39.14%), post-traumatic facial palsy (n = 21, 30.43%), chronic otitis media (n = 14, 20.29%) and iatrogenic (postsurgery or radiotherapy) (n = 7, 10.14%). There was no significant difference in the primary or secondary HFS in regard to age, sex, side affected, initial involvement of the upper or lower half of the face, presence of clicking in the ear and presence of hypertension. However, hearing loss [p \ 0.001; OR 0.28 (95% CI 0.13–0.57)], weakness of the upper facial muscles [p = 0.028; OR 0.54 (95% CI 0.31–0.94)] and lower facial muscles [p = 0.005; OR 0.36 (95% CI 0.21–0.63)], and preferential involvement of the orbicularis oculi (p = 0.045) and frontalis muscle (p = 0.013) were significantly more common among secondary HFS cases as compared to primary cases. Worsening with reading was significantly more common in primary HFS cases (p = 0.032) [OR 2.18 (95% CI 1.06–4.51)]. The other aggravating and relieving factors were almost equally seen between the two groups (Table 2). Presence of hypertension did not correlate with the side of HFS, other clinical variables, severity of spasm or relieving or aggravating factors. Severity of spasm was positively associated with duration of disease (p \ 0.001)
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Table 1 Demographic and clinical features among primary and secondary HFS patients Feature
Total (n = 321)
Primary HFS (n = 252)
Secondary HFS (n = 69)
Age (SD) mean years
46.01 (11.82)
46.07 (11.91)
45.81 (11.59)
P value; primary vs. secondary 0.436
Age at onset (SD) mean years
42.60 (11.77)
42.58 (11.95)
42.68 (11.17)
0.872
Duration of HFS mean (SD) months
43.15 (44.63)
43.15 (46.05)
41.78 (39.27)
0.564
Sex 50.78
52.38
44.93
0.273
L:R, n
Males (%)
175:145 (1 case bilateral)
138:113
37:32
0.959
Onset, n (%) Upper face
221 (68.85)
169 (67.06)
52 (75.36)
Lower face
17 (5.29)
16 (6.34)
1 (1.44)
Simultaneous
83 (25.86)
67 (26.58)
16 (23.19)
Interval between upper and lower. mean (SD) months
19.27 (22.81)
19.66 (23.90)
17.84 (18.21)
Clicking in ipsilateral ear, n (%)
73 (22.74)
54 (21.43)
19 (27.54)
0.237
Hearing loss ipsilateral ear, n (%)
36 (11.21)
20 (7.93)
16 (23.19)
\0.001
Hypertension, n (%)
67 (20.9)
56 (22.2)
11 (15.9)
0.255
0.198
Weakness of facial muscles Weakness upper face, n (%)
100 (31.2)
71 (28.2)
29 (42)
0.028
Weakness lower face, n (%)
96 (29.9)
66 (26.2)
30 (43.5)
0.005
Normal (CT/MRI), n (%)
135 (82.8)
98 (80.99)
37 (88.09)
Ecstatic vessel, n (%)
13 (7.98)
10 (8.26)
3 (7.14)
Imaging (163)
Severity, n (%)
0.109
0 Not affected, Normal 1 Very mild, occasional twitching, not disturbing
29 (9.0)
27 (10.7)
2 (2.9)
2 Mild, mildly disturbing
157 (48.9)
122 (48.4)
35 (50.7)
3 Mod. severe, frequent blinking, mildly affecting function
122 (38.0)
91 (36.3)
31 (44.9)
4 Very severe, prolonged spasm, severely affecting function
12 (3.7)
11 (4.4)
1 (1.4)
Table 2 Triggers and relieving factors in primary and secondary cases of HFS Trigger Eating, n (%) Speaking, n (%)
Total (n = 321) 77 (23.98) 121 (37.69)
Primary HFS (n = 252) 65 (25.8) 97 (38.5)
Secondary HFS (n = 69)
P
12 (17.4) 24 (34.8)
0.148 0.573
Going out in sun, n (%)
75 (23.36)
62 (24.6)
13 (18.8)
0.316
Reading, n (%)
78 (24.3)
68 (27)
10 (14.5)
0.032
61 (19.0)
53 (21)
8 (11.6)
0.077
Stress, n (%)
Watching TV, n (%)
144 (44.86)
119 (47.2)
25 (36.2)
0.104
People watching, n (%)
119 (37.07)
100 (39.7)
19 (27.5)
0.064
142 (44.24)
110 (43.7)
32 (46.4)
0.686
Relieving factors Sleep, n (%) Talking, n (%) Eating, n (%)
6 (1.87)
6 (2.4)
0
8 (3.2)
Wearing dark glasses, n (%)
18 (5.6)
13 (5.2)
5 (7.2)
0.504
Reading, n (%)
10 (3.1)
9 (3.6)
1 (1.4)
0.369
and presence of weakness of facial muscles (p \ 0.001). The cases of HFS following surgery, trauma or Bell’s palsy were not statistically different from each other with respect
2 (2.9)
0.196
10 (3.1)
0.907
to side, initial involvement of the upper or lower face, clicking in the ear, precipitating/relieving factors, or muscles involved.
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Discussion In India, botulinum toxin is not easily available because of cost constraints and the paucity of experts providing therapy with botulinum toxin in non-teaching hospitals. Only a handful of movement disorder specialists provides treatment of HFS with botulinum toxin, and ours is one of the largest centers in India providing this service. We are, therefore, in an advantageous position to see a large number of such patients from a large geographical area who need this therapy but have not been exposed to it. Hence, we were able to examine these patients before they had ever been exposed to it. Hemifacial spasm is seen almost in equal proportions between men and women, though it has been reported to be slightly more common in females, and two large hospitalbased studies reported a male-to-female ratio of 1:1.9 [10] and 1:1.6 [11] in primary HFS cases. Absence of female preponderance in our series may be a reflection of reluctance of women to seek medical consultations for equally severe medical disorders, as we have observed for Parkinson’s disease in India [13]. Although several authors have observed the left side to be more commonly affected by HFS [10, 11], this was not the case in our series. The reason for this predominance is attributed to the higher frequency of congenital anomalies on the left side of the vertebro-basilar arterial system, resulting in a higher frequency of ectatic and anomalous vessels on the left side, leading to pressure on the facial nerve root-exit zone [14]. However, it must be emphasized that none of the studies, including the present one, have been able to find a statistically significant difference between the two sides or demonstrated an aberrant vessel in the majority of cases of HFS. Primary HFS cases were approximately four times more common as compared to secondary HFS, a finding similar to the results of two other larger series [10, 11]. The onset of symptoms in the upper face, i.e., with involvement of the orbicularis oculi, is reported more frequently as compared to the lower face. Upper face involvement is known to be a characteristic feature of HFS [15]. Involvement of the upper face is reported to be more common in primary HFS, and simultaneous upper and lower involvement has been suggested in post-paralytic cases of HFS [11]. However, we did not observe this difference in our cases. Anatomically, there is somatotopic organization in the nucleus, fascicles and fibers of the facial nerve, with those innervating the lower face represented laterally, the upper face dorsally, and the platysma and posterior auricular muscles medially [16, 17]. The anterior inferior cerebellar artery (AICA) is known to be the most common vessel involved in compression of the root-exit zone of the facial nerve leading to HFS [17]. A
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study reporting on the relationship between the AICA and the VII–VIIIth nerve complex observed the AICA to be located dorsally to the VII–VIIIth nerve complex in 35% of patients [18]. In another series comprising 31 cases of HFS treated with microvascular decompression, more severe involvement of the upper face was observed with vessels compressing the cephalic aspect of the nerve [19]. This finding provides an answer to our observation of selective, earlier, more frequent and more severe upper facial muscle involvement compared to the lower facial muscles. We observed latency of approximately 20 months between involvement of muscles of the upper and lower face, which may be attributed to the insidious and progressive compression of the more ventral and caudal part of the VII– VIIIth nerve complex, although we have no evidence to support this statement. Facial weakness in primary HFS has been described [20], but no large series have prospectively assessed power in the muscles at first examination. As emphasized earlier, patients treated with botulinum toxin have residual weakness in the treated muscles that lasts several months; hence, power assessment can be fallacious if the patient has received botulinum toxin previously. However, we were privileged in this respect and saw a large majority of patients who were botulinum toxin naive. Facial weakness observed by us could have been due to associated facial fascicular demyelination. Weakness of facial muscles was significantly correlated with the severity and duration of disease (p \ 0.001). It is our postulate that repeated pressure of the vascular loop over the facial nerve would have caused mild but definite demyelination of the nerve, leading to weakness of the muscles. Presence of ear clicking on the ipsilateral side is interesting, and we believe this is due to clonic contraction of the stapedius muscle similar to clonic movement of other facial muscles leading to rarefaction and compression in the middle ear. Interestingly, there was mention of a clicking sound in facial spasms by Gowers in 1898, but this was attributed to palatal spasm [21]. The triggers/aggravating factors we have identified emphasize the episodic nature of the spasms, and as in all other paroxysmal disorders, control of triggers can be a useful therapeutic intervention. Stress as a trigger has been identified in some earlier studies [10]; other triggers that we describe have not been reported in other studies so far [10, 11]. Reading as an aggravating factor was found to be significantly more common in primary HFS cases. We find it hard to provide a physiological explanation for this difference in primary and secondary HFS cases because of the complex process involved in reading, including visual and mental tasks as well as physical changes in posture. Similarly, aggravation by watching television was also much more common in the primary HFS group (p = 0.07).
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Probably tasks requiring visual stimulation and concentration are important triggers in primary as compared to secondary cases. Hypertension has been reported to be present in 38.9–40% [11, 14] of cases of primary HFS. We observed hypertension in 22.22% of the primary HFS patients, a rate slightly lower than reported in previous studies. Hypertension was reported in 40% of HFS cases as compared to 25% in controls, with a strong association with left-sided HFS [14]. The authors attributed this association to additional neurovascular compression of the left VLM at the root-entry zone of cranial nerves IX and X. We did not observe any association between hypertension and the side of HFS. Asians with HFS living in the US have been shown to have a higher prevalence of hypertension, with 35.7% being hypertensive compared to 18.5% of Caucasians [9]. To conclude, the present study is the largest study on HFS from Asia and the only one that prospectively assessed HFS patients on their first visit. Interesting observations of this study are lack of female preponderance, presence of clicking in the ipsilateral ear and facial weakness. Some hitherto unreported triggers/aggravating and relieving factors have been identified. We did not find any correlation between left side of HFS and hypertension. Longer duration of HFS was associated with more severe spasms and weakness of muscles. Acknowledgments The authors acknowledge with thanks the statistical analysis and help by Ms. M. Kalaivani, M.Sc. (Biostatistics), Department of Biostatistics, and residents of the Department of Neurology, who collected patient data at the All India Institute of Medical Sciences, New Delhi, India. Conflicts of interest
None.
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